Virally mediated Kcnq1 gene replacement therapy in the immature scala media restores hearing in a mouse model of human Jervell and Lange-Nielsen deafness syndrome

EMBO Molecular Medicine

Volumn 7, Issue 8, 2015, Pages 1077-1086

  Chang, Qing ^a   Wang, Jianjun ^a   Li, Qi ^a,b   Kim, Yeunjung ^a   Zhou, Binfei ^a   Wang, Yunfeng ^c   Li, Huawei ^c   Lin, Xi ^c  

^a EMORY UNIVERSITY SCHOOL OF MEDICINE   (United States)

^b FIRST MILITARY MEDICAL UNIVERSITY   (China)

^c FUDAN UNIVERSITY   (China)

Author keywords

Gene therapy; Hearing restoration; Jervell and Lange Nielsen syndrome; Kcnq1 null mice; Virus

Indexed keywords

PARVOVIRUS VECTOR; POTASSIUM CHANNEL KCNQ1; KCNQ1 PROTEIN, MOUSE;

ADULT; ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL MODEL; ANIMAL TISSUE; ARTICLE; COCHLEA DUCT; CONTROLLED STUDY; ENDOLYMPH; EVOKED BRAIN STEM AUDITORY RESPONSE; FEMALE; GENE CASSETTE; GENE EXPRESSION; GENE MUTATION; GENE REPLACEMENT THERAPY; HAIR CELL; HEARING; JERVELL AND LANGE-NIELSEN SYNDROME; KCNQ1 GENE; MALE; MOUSE; NONHUMAN; PHENOTYPE; PRIORITY JOURNAL; SPIRAL GANGLION; STRIA VASCULARIS; VIRAL GENE DELIVERY SYSTEM; ANIMAL; DEAFNESS; DEPENDOPARVOVIRUS; DISEASE MODEL; GENE THERAPY; GENETIC TRANSDUCTION; GENETICS; JERVELL-LANGE NIELSEN SYNDROME; KNOCKOUT MOUSE; PHYSIOLOGY; PROCEDURES; TRANSGENE; TREATMENT OUTCOME;

ANIMALS; COCHLEAR DUCT; DEAFNESS; DEPENDOVIRUS; DISEASE MODELS, ANIMAL; GENE EXPRESSION; GENETIC THERAPY; JERVELL-LANGE NIELSEN SYNDROME; KCNQ1 POTASSIUM CHANNEL; MICE; MICE, KNOCKOUT; TRANSDUCTION, GENETIC; TRANSGENES; TREATMENT OUTCOME;

EID: 84938423524     PISSN: 17574676     EISSN: 17574684     Source Type: Journal    
DOI: 10.15252/emmm.201404929     Document Type: Article

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